Phenotypic Analyses of the HD Transgenic Minipig Model (A11609)
Ellederová, Zdeňka
2018 - English
The transgenic Huntington's disease minipigs (TgHD) express N‐terminal part of human mutated huntingtin (124Q) under the control of human huntingtin promoter. The founder animal, born in 2009, gave birth to four subsequent generations with an equal contribution of wild‐type (WT) and transgenic (TgHD) piglets in all litters. The model is being used for preclinical huntingtin lowering studies. Here we take different non-invasive and invasive approaches, some of which are unique for large animal models, to study the phenotype development comparing WT and TgHD siblings. We show gradual progression of the disease in these TgHD animals. Moreover, some biomarkers were identified. These markers could serve for monitoring of organism response to HD treatment to assess efficacy and safety in preclinical studies prior to human clinical trials.
Keywords:
minipigAvailable at various institutes of the ASCRPhenotypic Analyses of the HD Transgenic Minipig Model (A11609)

The transgenic Huntington's disease minipigs (TgHD) express N‐terminal part of human mutated huntingtin (124Q) under the control of human huntingtin promoter. The founder animal, born in 2009, gave ...

Oxidative stress in primary porcine fibroblasts expressing mutated huntingtin
Šmatlíková, Petra; Askeland, G.; Vaškovičová, Michaela; Klíma, Jiří; Motlík, Jan; Eide, L.; Ellederová, Zdeňka
2017 - English
Molecular events, such as protein aggregation, mitochondrial dysfunction, and transcriptional dysregulation have been linked to Huntington’s disease (HD) pathogenesis. Oxidative stress has been considered as one of the key players in disease progression. Though, it is still not clear whether oxidative stress causes HD, or if it is a consequence of other primary events.
Keywords:
huntingtinAvailable at various institutes of the ASCROxidative stress in primary porcine fibroblasts expressing mutated huntingtin

Molecular events, such as protein aggregation, mitochondrial dysfunction, and transcriptional dysregulation have been linked to Huntington’s disease (HD) pathogenesis. Oxidative stress has been ...

Role of planar cell polarity pathway in ameloblastoma with focus on frizzled 6
Putnová, Barbora; Putnová, Iveta; Hrubá, Eva; Dosedělová, Hana; Štembírek, Jan; Daněk, Z.; Buchtová, Marcela
2017 - English
Ameloblastoma is the most common epithelial odontogenic tumour in the oral cavity. Its progression is usually slow and the surface is covered by an oral mucosa with physiologic appearance. These tumours do not cause any pain to the patient and therefore it is difficult to diagnose them on time and the first signs are often missed by clinician leading to late diagnosis.
Keywords:
ameloblastomaAvailable at various institutes of the ASCRRole of planar cell polarity pathway in ameloblastoma with focus on frizzled 6

Ameloblastoma is the most common epithelial odontogenic tumour in the oral cavity. Its progression is usually slow and the surface is covered by an oral mucosa with physiologic appearance. These ...

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